Abdel Megeed Nagi Salma, Ibrahim Elashmawy Mai, E Elashkar Amany, Zaeim Hafez Mohamed, A E Emara Ashraf, Mohammad Abdelhay Osama, A B Fouda Albayoumi, AbdelAziz Doma Mohamed, Mohamed Awad Ahmad, Mohammed Saba Ahmed, Abdelrahman Ahmed Hesham, Mohamed Gad Allah Ahmed, Mahmoud Abdelraheem Fatma, A Gad Mohamed, A Soliman Mohamad, I Abdalrhman Tamer, Hassaan Awad Khaled, A K M El-Lebedy Ismael, M Abdelnaser Mostafa, Z Abdel Kareem Mohammed, Fekry Hassan Marwa, Sobhy Menshawy Khalifa Shymaa
Faculty of Medicine, King Salman International University, South Sinai, Egypt.
Pediatric Hepatology, Gastroenterology, and Nutrition Department, National Liver Institute, Menoufia University, Shebin ElKom, Egypt.
Clin Exp Pediatr. 2025 Aug 6. doi: 10.3345/cep.2025.01011.
Clinical, biochemical, histological, and immunological indicators are frequently used to diagnose autoimmune hepatitis (AIH), a chronic inflammatory liver disease affecting children. Wilson disease, which resembles AIH, is mainly evaluated using serum ceruloplasmin and copper levels. However, changes in these biomarkers have also been observed in AIH, raising the question of whether they could be useful for evaluating children with AIH.
When selecting a treatment plan and estimating the long-term prognosis of patients with AIH, assessing the liver fibrosis stage is crucial. It is also crucial to identify noninvasive indicators of liver fibrosis, for which ceruloplasmin has been suggested as a biomarker in several liver diseases. Therefore, this study aimed to investigate the potential significance of serum ceruloplasmin and copper levels for identifying liver fibrosis in children with AIH.
One hundred children with AIH treated at Menoufia University's National Liver Institute Pediatric Hepatology, Gastroenterology, and Nutrition Department were enrolled. The duration of the study was 5 years (February 2020 to February 2025). The patients' histopathological, radiographic, laboratory, and clinical data were collected. We used the revised score to diagnose AIH. A Beckman Coulter AU480 chemistry analyzer was used to measure serum copper, while an enzyme-linked immunosorbent assay was used to measure serum ceruloplasmin.
Serum ceruloplasmin levels were considerably lower in patients with advanced fibrosis (F3-4) than in those without advanced fibrosis (F0-2) (P<0.001). However, in patients with extensive fibrosis, the serum copper levels were considerably elevated (P<0.001). Compared to serum copper level, which had an area under a curve of 0.939 (95% confidence interval [CI], 0.887-0.991; P<0.001) and a cutoff of >24.7 mg/dL (90.8% sensitivity, 66.9% specificity), ceruloplasmin level had an area under a curve of 0.945 (95% CI, 0.889-1.00; P<0.001), suggesting that it could be a useful tool for the detection of advanced liver fibrosis in children.
To estimate the long-term prognosis of patients with AIH, it is crucial to assess liver fibrosis stage. It is crucial to identify noninvasive indicators of liver fibrosis, for which ceruloplasmin has been suggested as a biomarker. Therefore, serum copper and ceruloplasmin levels may provide important information for the identification of advanced liver fibrosis in children with AIH.
临床、生化、组织学和免疫学指标常用于诊断自身免疫性肝炎(AIH),这是一种影响儿童的慢性炎症性肝病。肝豆状核变性与AIH相似,主要通过血清铜蓝蛋白和铜水平进行评估。然而,在AIH中也观察到了这些生物标志物的变化,这就提出了它们是否可用于评估AIH患儿的问题。
在选择AIH患者的治疗方案和评估其长期预后时,评估肝纤维化阶段至关重要。识别肝纤维化的非侵入性指标也很关键,在几种肝病中,铜蓝蛋白已被提议作为一种生物标志物。因此,本研究旨在探讨血清铜蓝蛋白和铜水平在识别AIH患儿肝纤维化方面的潜在意义。
招募了在曼苏拉大学国家肝脏研究所儿科肝病、胃肠病和营养科接受治疗的100例AIH患儿。研究持续时间为5年(2020年2月至2025年2月)。收集了患者的组织病理学、影像学、实验室和临床数据。我们使用修订后的评分来诊断AIH。使用贝克曼库尔特AU480化学分析仪测量血清铜,同时使用酶联免疫吸附测定法测量血清铜蓝蛋白。
晚期纤维化(F3 - 4)患者的血清铜蓝蛋白水平显著低于无晚期纤维化(F0 - 2)的患者(P<0.001)。然而,在广泛纤维化的患者中,血清铜水平显著升高(P<0.001)。与血清铜水平相比,其曲线下面积为0.939(95%置信区间[CI],0.887 - 0.991;P<0.001),截断值>24.7mg/dL(敏感性90.8%,特异性66.9%),铜蓝蛋白水平的曲线下面积为0.945(95%CI,0.889 - 1.00;P<0.001),这表明它可能是检测儿童晚期肝纤维化的有用工具。
为了评估AIH患者的长期预后,评估肝纤维化阶段至关重要。识别肝纤维化的非侵入性指标很关键,铜蓝蛋白已被提议作为一种生物标志物。因此,血清铜和铜蓝蛋白水平可能为识别AIH患儿的晚期肝纤维化提供重要信息。